The Number of Surgical Interventions and Specialists Involved in the Management of Patients with Neurofibromatosis Type I: A 25-Year Analysis

Abstract

Objective: In this study, we aim to present a single institution’s 25-year experience of employing a comprehensive multidisciplinary team-based surgical approach for treating patients with NF-1. Summary Background Data: All patients (n = 106) with a confirmed diagnosis of NF-1 who were treated using a multidisciplinary surgical treatment algorithm at Chang Gung Memorial Hospital between 1994 and 2019 were retrospectively enrolled. Patients were categorized into groups according to the anatomy involved (craniofacial and noncraniofacial groups) and the type of clinical presentation (plexiform and cutaneous neurofibromas groups) for comparative analysis. Methods: The number of surgical interventions and number of specialists involved in surgical care were assessed. Results: Most of the patients exhibited craniofacial involvement (69.8%) and a plexiform type of NF-1 (58.5%), as confirmed through histology. A total of 332 surgical interventions (3.1 ± 3.1 procedures per patient) were performed. The number of specialists involved in surgical care of the included patients was 11 (1.6 ± 0.8 specialists per patient). Most of the patients (62.3%) underwent two or more surgical interventions, and 40.6% of the patients received treatment from two or more specialists. No significant differences were observed between the craniofacial and noncraniofacial groups in terms of the average number of surgical interventions (3.3 ± 3.2 vs. 2.7 ± 2.7, respectively) and number of specialists involved (1.7 ± 0.9 vs. 1.4 ± 0.6). Patients with plexiform craniofacial involvement underwent a significantly higher average number of surgical interventions (4.3 ± 3.6 vs. 1.6 ± 1.1; p < 0.001) and received treatment by more specialists (1.9 ± 0.9 vs. 1.2 ± 0.5; p < 0.001) compared with those having cutaneous craniofacial involvement. Conclusions: In light of the potential benefits of employing the multidisciplinary team-based surgical approach demonstrated in this study, such an approach should be adopted to provide comprehensive individualized care to patients with NF-1.

Neurofibromatosis type 1-associated plexiform neurofibromas of the neck: topography of lesions and surgical treatment data of 69 patients

“…In addition, the tumor distribution was not classified according to dermatomes or anatomic units. Earlier studies suggest that head and neck PNF are very common tumor sites in NF1 patients requiring surgical intervention [ 3 , 16 , 17 , 35 , 36 ].…”

Neurofibromatosis Type 1-Associated Plexiform Neurofibromas of the Face and Adjacent Head Regions: Topography of Lesions and Surgical Treatment Data of 179 Patients

“…Topographic analysis of craniofacial PNF tumor spreads is an important contribution to assessing the treatment needs of these patients. Among NF1-associated PNF, craniofacial PNF requires the most surgical treatment [ 3 , 13 ]. Both classifications of the facial surface used for topographic evaluation show PNST frequency symmetry illustrated by the tumor-affected regions marked with the same intensity of color on both sides, namely eyes/orbits, temples, cheeks, and lateral forehead.…”

“…Likewise, the diagnosis of PNF was not further specified. Other studies suggest that craniofacial PNF is among the most common tumor sites in NF1 patients requiring surgical intervention [ 3 , 13 ]. However, from clinical judgment, the differences between PNF and diffuse neurofibroma appear to be gradual (Table 2 ).…”

Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1

“…Diffuse PN spread extensively along connective tissue and surround normal structures with indistinct borders, and nodular PN form firm and round tumors and often present with multiple discrete tumors arising from peripheral nerves [ 6 , 7 , 8 ]. Several surgical techniques have been proposed for diffuse PN to decrease intraoperative bleeding and facilitate tumor excision [ 9 , 10 , 11 , 12 , 13 ]. These include electrosurgical procedures, adhesive or thrombogenic substances, or intravascular embolization prior to surgery.…”